Overview

  • Product nameAnti-PSAP antibody
    See all PSAP primary antibodies
  • Description
    Rabbit polyclonal to PSAP
  • Tested applicationsSuitable for: ELISA, ICC/IF, WB, IP, IHC-Frmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    A synthetic peptide corresponding to an internal sequence of human PSAP.

  • Positive control
    • HepG2 whole cell lysate

Properties

Associated products

Applications

Our Abpromise guarantee covers the use of ab68466 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ELISA Use a concentration of 0.01 - 0.1 µg/ml.
ICC/IF Use a concentration of 1 µg/ml.
WB Use a concentration of 0.1 - 1 µg/ml. Detects a band of approximately 70 kDa (predicted molecular weight: 58 kDa).
IP Use a concentration of 2 - 5 µg/ml.
IHC-Fr Use at an assay dependent concentration. See Abreview.

Target

  • FunctionThe lysosomal degradation of sphingolipids takes place by the sequential action of specific hydrolases. Some of these enzymes require specific low-molecular mass, non-enzymic proteins: the sphingolipids activator proteins (coproteins).
    Saposin-A and saposin-C stimulate the hydrolysis of glucosylceramide by beta-glucosylceramidase (EC 3.2.1.45) and galactosylceramide by beta-galactosylceramidase (EC 3.2.1.46). Saposin-C apparently acts by combining with the enzyme and acidic lipid to form an activated complex, rather than by solubilizing the substrate.
    Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). Saposin-B forms a solubilizing complex with the substrates of the sphingolipid hydrolases.
    Saposin-D is a specific sphingomyelin phosphodiesterase activator (EC 3.1.4.12).
  • Involvement in diseaseDefects in PSAP are the cause of combined saposin deficiency (CSAPD) [MIM:611721]; also known as prosaposin deficiency. CSAPD is due to absence of all saposins, leading to a fatal storage disorder with hepatosplenomegaly and severe neurological involvement.
    Defects in PSAP saposin-B region are the cause of leukodystrophy metachromatic due to saposin-B deficiency (MLD-SAPB) [MIM:249900]. MLD-SAPB is an atypical form of metachromatic leukodystrophy. It is characterized by tissue accumulation of cerebroside-3-sulfate, demyelination, periventricular white matter abnormalities, peripheral neuropathy. Additional neurological features include dysarthria, ataxic gait, psychomotr regression, seizures, cognitive decline and spastic quadriparesis.
    Defects in PSAP saposin-C region are the cause of atypical Gaucher disease (AGD) [MIM:610539]. Affected individuals have marked glucosylceramide accumulation in the spleen without having a deficiency of glucosylceramide-beta glucosidase characteristic of classic Gaucher disease, a lysosomal storage disorder.
    Defects in PSAP saposin-A region are the cause of atypical Krabbe disease (AKRD) [MIM:611722]. AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease.
    Note=Defects in PSAP saposin-D region are found in a variant of Tay-Sachs disease (GM2-gangliosidosis).
  • Sequence similaritiesContains 2 saposin A-type domains.
    Contains 4 saposin B-type domains.
  • Post-translational
    modifications
    This precursor is proteolytically processed to 4 small peptides, which are similar to each other and are sphingolipid hydrolase activator proteins.
    N-linked glycans show a high degree of microheterogeneity.
    The one residue extended Saposin-B-Val is only found in 5% of the chains.
  • Cellular localizationLysosome.
  • Information by UniProt
  • Database links
  • Alternative names
    • A1 activator antibody
    • Cerebroside sulfate activator antibody
    • Co-beta-glucosidase antibody
    • Component C antibody
    • CSAct antibody
    • Dispersin antibody
    • GLBA antibody
    • Glucosylceramidase activator antibody
    • Proactivator polypeptide antibody
    • Proactivator polypeptide precursor antibody
    • Prosaposin (sphingolipid activator protein 1) antibody
    • prosaposin (variant Gaucher disease and variant metachromatic leukodystrophy) antibody
    • Prosaposin antibody
    • Protein A antibody
    • Protein C antibody
    • PSAP antibody
    • SAP-1 antibody
    • SAP-2 antibody
    • SAP_HUMAN antibody
    • SAP1 antibody
    • Saposin A antibody
    • Saposin B antibody
    • Saposin B Val antibody
    • Saposin C antibody
    • Saposin D antibody
    • Saposin-D antibody
    • Saposins antibody
    • Sgp1 antibody
    • Sphingolipid activator protein 1 antibody
    • Sphingolipid activator protein 2 antibody
    • Sulfated glycoprotein 1 antibody
    • Sulfatide/GM1 activator antibody
    see all

Anti-PSAP antibody images

  • Anti-PSAP antibody (ab68466) at 1/500 dilution + HepG2 whole cell lysate

    Predicted band size : 58 kDa
    Observed band size : 70 kDa (why is the actual band size different from the predicted?)
  • ICC/IF image of ab68466 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab68466, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

References for Anti-PSAP antibody (ab68466)

This product has been referenced in:
  • Lundius EG  et al. GPR37 protein trafficking to the plasma membrane regulated by prosaposin and GM1 gangliosides promotes cell viability. J Biol Chem 289:4660-73 (2014). WB ; Mouse . Read more (PubMed: 24371137) »
  • Hu F  et al. Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin. Neuron 68:654-67 (2010). WB ; Mouse . Read more (PubMed: 21092856) »

See all 3 Publications for this product

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Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (SH-SY5Y cells)
Loading amount 100 µg
Specification SH-SY5Y cells
Gel Running Conditions Reduced Denaturing (12% tris tricine)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 25°C
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Abcam user community

Verified customer

Submitted May 27 2011

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Mouse Cell lysate - whole cell (Mouse embryonic fibroblast)
Loading amount 100 µg
Specification Mouse embryonic fibroblast
Gel Running Conditions Reduced Denaturing (12% tricine)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 25°C
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Abcam user community

Verified customer

Submitted May 27 2011

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (cos7 cells)
Loading amount 20 µg
Specification cos7 cells
Gel Running Conditions Reduced Denaturing (10)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 22°C
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Verified customer

Submitted Jul 08 2010

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunohistochemistry (Frozen sections)
Sample Rat Tissue sections (Whole embryo)
Specification Whole embryo
Fixative Paraformaldehyde
Permeabilization Yes - Ethanol
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
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Verified customer

Submitted May 01 2009

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"